Top

Child's Nervous System

Published in:

01-08-2012 | Review Paper

Cerebral haemorrhage in the presence of primary childhood central nervous system vasculitis—a review

Authors: Karin Pistracher, Verena Gellner, Stefan Riegler, Bernadette Schökler, Michael Scarpatetti, Senta Kurschel

Published in: Child's Nervous System | Issue 8/2012

Abstract

Purpose

The aim of the study was to evaluate frequency and clinical relevance of haemorrhagic events associated with primary angiitis of the central nervous system in childhood (cPACNS), a rare but increasingly recognized disease with varying clinical presentations.

Method

A systematic literature review from 1990 onwards was conducted to identify reported cases of cPACNS.

Results

A total of 110 paediatric patients met the inclusion criteria. The median age was 9.5 years. Seven children (7/110, 6.4 %) demonstrated cerebral haemorrhage. Death occurred only in children with cerebral haemorrhage (4/110, 3.6 %); both a sudden and prolonged course of disease was observed.

Conclusion

PACNS is a rare disease and the occurrence of haemorrhage with this condition is even rarer; however, the risk of an unfavourable outcome under these circumstances seems to be increased. PACNS adds to the list of differential diagnoses of intracerebral haemorrhage in the paediatric population.

Abe T, Maruyama T, Nagai Y, Kamida T, Wakabayashi Y, Ishii K, Fujiki M, Kobayashi H (2007) Severe postoperative vasculitis of the central nervous system in a child with arteriovenous malformation: case report. Surg Neurol 68:317–321PubMedCrossRef

Ay H, Sahin G, Saatci I, Söylemezoğlu F, Saribaş O (2002) Primary angiitis of the central nervous system and silent cortical hemorrhages. AJNR Am J Neuroradiol 23:1561–1563PubMed

Benseler SM (2006) Central nervous system vasculitis in children. Curr Rheumatol Rep 8:442–449PubMedCrossRef

Benseler SM, deVeber G, Hawkins C, Schneider R, Tyrrell PN, Aviv RI, Armstrong D, Laxer RM, Silverman ED (2005) Angiography-negative primary central nervous system vasculitis in children. Arthritis Rheum 52:2159–2167PubMedCrossRef

Benseler SM, Silverman E, Aviv RI, Schneider R, Armstrong D, Tyrrell PN, deVeber G (2006) Primary central nervous system vasculitis in children. Arthritis Rheum 54:1291–1297PubMedCrossRef

Berlit P (2005) Zerebrale Vaskulitis. In: Diener HC, Putzki N, Berlit P, Hacke W, Hufnagel A, Hufschmidt A, Mattle H, Meier U, Oertel WH, Reichmann H, Rieckmann P, Schmutzhard E, Wallesch CW, Weller M; Kommission „Leitlinien der Deutschen Gesellschaft für Neurologie“ (eds) Leitlinien für Diagnostik und Therapie in der Neurologie, 3. Auflage. Thieme, Stuttgart, pp 268–283

Bitter KJ, Epstein LG, Melin-Aldana H, Curran JG, Miller ML (2006) Cyclophosphamide treatment of primary angiitis of the central nervous system in children: report of 2 cases. J Rheumatol 33:2078–2080PubMed

Brunner J, Prelog M, Zimmerhackl LB (2007) Primäre ZNS-Vaskulitis bei Kindern: Differentialdiagnose eines zerebralen Insultes. J Neurol Neurochir Psychiatr 8:7–8

Budzilovich GN, Feigin I, Seigel H (1963) Granulomatous angiitis of the nervous system. Arch Neurol 19:250–256

10.

Calabrese LH, Furlan AJ, Gragg LA, Ropos TJ (1992) Primary angiitis of the central nervous system: diagnostic criteria and clinical approach. Cleve Clin J Med 59:293–306PubMed

11.

Carolei A, Sacco S (2003) Central nervous system vasculitis. Neurol Sci 24:S8–S10PubMedCrossRef

12.

Cekinmez EK, Cengiz N, Erol I, Kizilkiliç O, Uslu Y (2009) Unusual cause of acute neurologic deficit in childhood: primary central nervous system vasculitis presenting with basilar arterial occlusion. Childs Nerv Syst 25:133–136PubMedCrossRef

13.

Cellucci T, Benseler SM (2010) Central nervous system vasculitis in children. Curr Opin Rheumatol 22:590–597PubMedCrossRef

14.

Cellucci T, Benseler SM (2010) Diagnosing central nervous system vasculitis in children. Curr Opin Pediatr 22:731–738PubMedCrossRef

15.

De Tiège X, Van Bogaert P, Aeby A, Salmon I, Parpal H, Poppe AY, Maris C, Lanthier S (2011) Primary angiitis of the central nervous system: neurologic deterioration despite treatment. Pediatrics 127:e1086–e1090PubMedCrossRef

16.

Elbers J, Benseler SM (2008) Central nervous system vasculitis in children. Curr Opin Rheumatol 20:47–54PubMedCrossRef

17.

Elbers J, Halliday W, Hawkins C, Hutchinson C, Benseler SM (2010) Brain biopsy in children with primary small-vessel central nervous system vasculitis. Ann Neurol 68:602–610PubMedCrossRef

18.

Gallagher KT, Shaham B, Reiff A, Tournay A, Villablanca JP, Curran J, Nelson MD Jr, Bernstein B, Rawlings DJ (2001) Primary angiitis of the central nervous system in children: 5 cases. J Rheumatol 28:616–623PubMed

19.

Greenan TJ, Grossman RI, Goldberg HI (1992) Cerebral vasculitis: MR imaging and angiographic correlation. Radiology 182:65–72PubMed

20.

Ho VB, Smirniotopoulos JG, Murphy FM, Rushing EJ (1992) Radiologic-pathologic correlation: hemangioblastoma. AJNR Am J Neuroradiol 13:1343–1352PubMed

21.

Hutchinson C, Elbers J, Halliday W, Branson H, Laughlin S, Armstrong D, Hawkins C, Westmacott R, Benseler SM (2010) Treatment of small vessel primary CNS vasculitis in children: an open-label cohort study. Lancet Neurol 9:1078–1084PubMedCrossRef

22.

Kamm C, Nägele T, Mittelbronn M, Schöning M, Melms A, Gasser T, Schöls L (2008) Primary central nervous system vasculitis in a child mimicking parasitosis. J Neurol 255:130–132PubMedCrossRef

23.

Kumar R, Wijdicks EFM, Brown RD Jr, Parisi JE, Hammond CA (1997) Isolated angiitis of the CNS presenting as subarachnoid haemorrhage. J Neurol Neurosurg Psychiatry 62:649–651PubMedCrossRef

24.

Lanthier S, Lortie A, Michaud J, Laxer R, Jay V, deVeber G (2001) Isolated angiitis of the CNS in children. Neurology 56:837–842PubMedCrossRef

25.

MacLaren K, Gillespie J, Shrestha S, Neary D, Ballardie FW (2005) Primary angiitis of the central nervous system: emerging variants. Q J Med 98:643–654CrossRef

26.

Matsell DG, Keene DL, Jimenez C, Humphreys P (1990) Isolated angiitis of the central nervous system in childhood. Can J Neurol Sci 17:151–154PubMed

27.

Nishikawa M, Sakamoto H, Katsuyama J, Hakuba A, Nishimura S (1998) Multiple appearing and vanishing aneurysms: primary angiitis of the central nervous system. J Neurosurg 88:133–137PubMedCrossRef

28.

Pomper MG, Miller TJ, Stone JH, Tidmore WC, Hellmann DB (1999) CNS vasculitis in autoimmune disease: MR imaging findings and correlation with angiography. AJNR Am J Neuroradiol 20:75–85PubMed

29.

Sen ES, Leone V, Abinun M, Forsyth R, Ramesh V, Friswell M, O’Callaghan F, Ramanan AV (2010) Treatment of primary angiitis of central nervous system in childhood with mycophenolate mofetil. Rheumatology 49:806–811PubMedCrossRef

30.

Shimizu N, Sugita K, Ishii M, Kawazoe Y, Miyamoto H, Niimi H (1994) A female case of isolated angiitis of the central nervous system. No To Hattatsu 26:38–43PubMed

31.

Siva A (2001) Vasculitis of the nervous system. J Neurol 248:451–468PubMedCrossRef

32.

Yaari R, Anselm IA, Szer IS, Malicki DM, Nespeca MP, Gleeson JG (2004) Childhood primary angiitis of the central nervous system: two biopsy-proven cases. J Pediatr 145:693–697PubMedCrossRef

33.

Zuber M, Blustajn J, Arquizan C, Arquizan C, Trystram D, Mas JL, Meder JF (1999) Angiitis of the central nervous system. J Neuroradiol 26:101–117PubMed

Title: Cerebral haemorrhage in the presence of primary childhood central nervous system vasculitis—a review
Authors: Karin Pistracher
Verena Gellner
Stefan Riegler
Bernadette Schökler
Michael Scarpatetti
Senta Kurschel
Publication date: 01-08-2012
Publisher: Springer-Verlag
Published in: Child's Nervous System / Issue 8/2012
Print ISSN: 0256-7040
Electronic ISSN: 1433-0350
DOI: https://doi.org/10.1007/s00381-012-1827-4

At a glance: The STEP trials

Springer Medicine

Cerebral haemorrhage in the presence of primary childhood central nervous system vasculitis—a review

Abstract

Purpose

Method

Results

Conclusion

At a glance: The STEP trials

Springer Medicine

Abstract

Purpose

Method

Results

Conclusion

Please log in to get access to this content

Other articles of this Issue 8/2012

Congenital brain tumors in a series of 56 patients

Endoscopic Third Ventriculostomy Success Score (ETVSS) predicting success in a series of 50 pediatric patients. Are the outcomes of our patients predictable?

Pediatric neurosurgery at the Hospital for Sick Children, The University of Toronto

Third ventriculostomy in infants younger than 1 year old

Targeted neurosurgical outreach: 5-year follow-up of operative skill transfer and sustainable care in Lima, Peru

The most important decision in decompressive craniectomy in pediatric traumatic brain injury: when not to perform it!